Management of Challenging Ebstein’s Anomaly Case in a Teenager Patient

Aasim M, Ul Mohsin A, Asad Khan M, Aslam Awan Z, Ghaffar R and Aziz R

Department of Cardiac Surgery, MTI-HMC Peshawar, Pakistan Department of Cardiology, MTI-HMC Peshawar, Pakistan

^*Correspondance to: Muhammad Aasim 

 PDF  Full Text DOI: 10.25107/2474-1647.3685

Abstract

Ebstein’s anomaly is a relatively rare form of congenital malformation of the tricuspid valve and right ventricular failure [1] with prevalence of 1/20,000 live births and accounts for 1% of all cases of congenital heart disease [2-4]. It was first described by Wilhelm Ebstein in 1866 [5]. Ebstein’s anomaly has a broad clinical spectrum depending on the severity of the tricuspid regurgitation; age of clinical presentation, hemodynamic variations and degree of right-to-left interatrial shunt, right ventricular functions and associated abnormalities. The cardinal symptoms in Ebstein’s anomaly are progressive cyanosis, right ventricular myopathy, arrhythmias, and sudden cardiac death. Many cases of Ebstein anomaly can be missed on routine Echocardiography and remain undiagnosed for longer time period which can delay definitive treatment in a significant symptomatic patient, by the time of diagnosis many patients develop advance heart failure having unstable hemodynamics specially in cases coexistence with arrhythmias leads to increase morbidity and mortality and many cardiac surgeons refuse their surgical intervention due to unstable hemodynamics. In such cases newly advanced specific cardiac modalities should be used 1st line for early diagnosis and prompt treatment where available.

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Cite the article

Aasim M, Ul Mohsin A, Asad Khan M, Aslam Awan Z, Ghaffar R, Aziz R. Management of Challenging Ebstein’s Anomaly Case in a Teenager Patient. Clin Surg. 2024; 9: 3685..